Multimodality treatment of a massive cervicothoracic lymphatic-venous malformation in a 13-year-old boy

Abstract

We report the multi-modality treatment of a massive cervicothoracic lymphatic-venous malformation (LVM) in a 13-year-old boy that was present at birth as a small cervical mass. At four years of age, sclerotherapy significantly reduced the size of the malformation but over subsequent years the mass increased dramatically in size and resulted in a consumptive coagulopathy and limited left shoulder function. Treatment with sildenafil was without effect. The consumptive coagulopathy improved with subcutaneous enoxaparin. A multi-disciplinary team excised part of the mass when he was 11 years old. After surgery, gradual enlargement of the mass and rise in d-dimer prompted treatment with sirolimus. Two sessions of sclerotherapy arrested enlargement of the mass. Multi-modality treatment resulted in a marked reduction of the LVM in the child. However, enlargement of the mass and further treatment is anticipated as a cure has not yet been found.

Keywords

• Lymphatic-venous malformation
• Kassabach-Merritt syndrome
• Treatment